Frontotemporal dementia (FTD) is the name given to dementia when it is due to progressive damage to the frontal and/or temporal lobes of the brain.
“It can be and often is really hard to tackle a behaviour directly with someone who’s not aware of the impact of their behaviour. “Some people will react aggressively towards their loved one because of something that’s happening in the environment. And then the question is: Can we identify what it is that triggers his response?
Frontotemporal dementia is estimated to be the second most common cause of dementia in younger people after Young Onset Alzheimer’s disease. Many, if not most, FTD suffers are misdiagnosed and are often treated for psychiatric disorders, depression, or Alzheimer’s itself.
What’s wrong with Jacques? Exploring frontotemporal dementia
By: Conor Burke in Clinical Focus, Top Stories October 24, 2019
https://www.agedcareinsite.com.au/2019/10/whats-wrong-with-jacques-exploring-frontotemporal-dementia/
Olivier Piguet knew something was wrong with his cousin Jacques. During a phone conversation, Olivier’s mother mentioned that Jacques, then a successful and well-liked optometrist in his local Swiss town, was acting peculiar. Suddenly rude to customers and falling out with his business partner, he was struggling to keep the business running. Olivier told his mother that this sounded like the early symptoms of frontotemporal dementia (FTD). This would have been classed as an amazing feat of diagnostic assessment were it not for the fact that Olivier had spent his life studying the disease as a research fellow and professor of clinical neuropsychology.
Jacques was lucky that his cousin, thousands of miles away in Australia, was familiar with these symptoms, as FTD can often go misdiagnosed for years, especially in people under 65 – like Jacques, then aged 49. FTD can often be diagnosed as depression or other mental illness as the initial symptoms can be similar.
“When some someone starts developing these symptoms, you don’t think dementia because dementia is something that happens in old age,” Piguet says.
“If you start developing these symptoms in your 30s, 40s, it takes about five years to get to a diagnosis. When you’re in your 70s it takes about half as long to get a diagnosis of dementia. So, 18 months, two years.”
FTD is a form of dementia that is a result of damage to the frontal and/or lobal areas of the brain, the area that is important for controlling voluntary behaviour, emotions and complex thought. Although FTD is a less common disease, approximately 15–20 per cent of all cases of dementia will have some FTD-related syndromes. It most commonly affects people in their 50s or 60s, compared with Alzheimer’s disease which manifests at a later age.
FTD can be divided into three sub-categories (although these sometimes overlap):
There is the behavioural variant and two language presentations –
“Progressive non-fluent aphasia is much more obvious because you have this difficulty with expressive language. They understand fine, but they want to produce the output; that’s where there’s a major problem. So you have all this distorted garbled speech.”
People with FTD will often present with one of the three sub-types initially and then develop other symptoms over time.
The ways we treat FTD are different as well. Unlike other types of dementia, it does not respond to medication, says Piguet.
“In terms of disease modifying treatments like drugs, there’s really nothing in the dementia space. The only drugs that are available will essentially boost the efficiency of the nerve cells that are left in the brain. So like drinking 10 cups of coffee essentially – it makes you more buzzy and aware. It helps these nerve cells work as best as they can, but it doesn’t stop the disease process. So progressively these nerve cells will still die, and the medications will stop working,” he says.
“In frontotemporal dementia, we don’t even have that. All the drug trials that have been going on in the past few years have been negative. One reason is because of these multiple pathologies – a drug is likely to target on one of these protein accumulations, not all of them.” However, like Alzheimer’s, there are ways in which people with FTD can train and exercise their brains to allay symptoms and slow decline. Attempting to alleviate behaviours is important for the carers and families of those with FTD.
- - - - - - - - - - - - - - - - - - - - - - - - - - - - - -
FTD refers to a group of rare neurological disorders mostly affecting the frontal and anterior temporal lobes of the brain.
These areas control “executive functions” such as
FTD usually develops between the ages of 35 and 75 affecting men and women about equally. FTD is believed to make-up only 3% of all dementia cases.
Some of the diseases classified as FTD include:
The frontal and temporal lobes handle the executive and language functions of the brain. When these brain regions are affected, many of the brain’s communication, sorting and coordination functions are impacted. This means that disability, behaviour disturbance and risk can be more severe in earlier stages of FTD than in other forms of dementia.
People with hyperactive or ritualistic behavioural presentations of FTD are often excluded or rejected from planned activity programs or social gatherings for people with dementia, because of a perceived inability to benefit or because their symptoms disrupt others in the group. It is important to consider FTD as a focal dementia, meaning that more medial and posterior functions of the brain such as memory and visual/spatial skills may be relatively spared for much of the disease course.
Research by neuroscientists at the University of Sydney has shown that people living with frontotemporal dementia – a form of younger-onset dementia – lose the ability to daydream and have no inner monologue. It is believed that those with healthy minds spend at least 50% of their waking lives daydreaming and allowing their mind to wander, and this train of internal thought has many positive benefits for overall mental health.
01 March 2019
Introspective thinking and daydreaming allow a person to analyse their own behavior and the behaviors of others as they reflect on past events and plan for future events in their mind. This type of thinking is one of the main catalysts for emotional and behavioral regulation and it is also associated with problem-solving skills and personal creativity.
Professor Muireann Irish, Associate Professor Brain and Mind Centre and School of Psychology at the University of Sydney noticed many interesting things throughout the study, but even though the study was obviously informative, the findings did reveal an upsetting reality. “Unfortunately, one of the things that we took from the study was just how sad these findings really are. Imagine not having the ability to daydream and escape the present moment. Imagine how limiting that is to a person,” said Professor Irish.
An FTD specific program may place more emphasis on short activities that require less concentration, activities that burn off excess energy and agitation with physical activity and more meaningful activities for a younger cohort. Similarly individualized assessment may find ways to engage individuals with symptoms of apathy.
How is carer burden different in FTD?
Carers of individuals with FTD are an often overlooked group. Importantly, carers are at risk of experiencing physical, emotional, and social difficulties while caring for someone with a condition such as FTD (Schulz and Martire 2004). The caregiver burden in FTD is greater than in other forms of dementia such as AD (Piguet, Hornberger et al. 2011), and is possibly related to the unique behavioural and cognitive symptoms of FTD. People with FTD often lose insight early in their disease process, creating significant barriers to the person accepting they have a problem, their willingness to seek diagnosis and to accept they need help and support. This greatly contributes to tensions in communication and care delivery as the person with FTD may resist support.
People with FTD will often lose decision making capacity and planning skills early and so carers of people with FTD tend to be supporting these functions for longer than in other types of dementia.
The U.S based website: www.ftdsupport.com offers an international chat forum for FTD carers specifically. Alzheimer’s Australia Vic has also evaluated a pilot chat forum for people with younger onset dementia and their carers.
FTD Online Support Forum is at http://www.ftdsupportforum.com/
The purpose of this online forum is to provide a place for safe and secure communications for people who have been diagnosed with FTD and those who care for loved ones with FTD.
Members are dedicated to supporting one another in a sensitive, respectful and sincere manner.
This forum also encourages increased awareness of FTD within the medical community and the general public, with the belief that knowledge will bring improved access to support networks that are emerging on the internet and elsewhere.
http://www.theaftd.org.au/about-afd/
Download toolkits at https://ecdc.org.au/ftd-toolkit
Note: by clicking on each heading, there is a relevant dropdown with a .pdf to download.
https://www.dementia.org.au/information/about-dementia/types-of-dementia/frontotemporal-dementia
The right and left frontal lobes at the front of the brain are involved in
The right and left temporal lobes at the two sides of the brain are involved in processing what we hear and understanding what we hear and see. Damage may lead to difficulty recognising objects or understanding or expressing language.
FTD is sometimes called frontotemporal lobar degeneration. It was first described 100 years ago by Arnold Pick and was previously referred to as Pick’s disease.
The symptoms of FTD depend on which areas of the brain are damaged. In contrast to Alzheimer’s disease, memory often remains unaffected in FTD, especially in the early stages. When the frontal lobes are affected first, the main changes are in personality and behaviour, and this is called behavioural-variant FTD. When the temporal lobes are affected first, there is a loss of language skills. There are two types of FTD where language is impaired – progressive nonfluent aphasia and semantic dementia.
Behavioural-variant frontotemporal dementia
In the frontal or behavioural variant of FTD, there are changes in the person’s behaviour, habits, personality and/or emotional responses. Symptoms vary from person to person depending on which areas of the frontal lobes are damaged. Some people with behavioural-variant FTD become very apathetic, while others become disinhibited.
Common symptoms can include:
Reduced ability to perform daily activities is an early feature. As the disease progresses, the person may become obsessive, or repeat patterns of movement or behaviours.
The Australian Fronto-Temporal Dementia Association (AFTDA)
Overlap between frontotemporal dementia and movement disorders
Most people with FTD do not experience significant movement or motor problems. However, motor symptoms can develop in the later stages of the illness and FTD can co-occur with other conditions characterised by motor symptoms.
These include motor neuron disease, corticobasal syndrome, progressive supranuclear palsy and FTDP-17 (FTD with parkinsonism linked to chromosome 17).
Is there treatment available?
There is not yet a cure for FTD, or any diseasemodifying treatment. However, various therapies can help with some of the symptoms. Secondary symptoms, such as depression, may be helped by medication.
Knowing more about FTD and why the person is behaving as they are can help people to cope with the disease.
Family members and carers can, with support, develop coping strategies to work around problems rather than trying to change the behaviour of the person with FTD.
Click here for information - like how to find them :-) on the following therapies:
Psychological therapies are important to help manage abnormal behaviour.
Speech therapy is of benefit to people with PNFA, particularly in helping to develop alternative communication methods.
The input of an Occupational therapist can help improve everyday functioning at home.
8 November 2019
“It can be and often is really hard to tackle a behaviour directly with someone who’s not aware of the impact of their behaviour. “Some people will react aggressively towards their loved one because of something that’s happening in the environment. And then the question is: Can we identify what it is that triggers his response?
Frontotemporal dementia is estimated to be the second most common cause of dementia in younger people after Young Onset Alzheimer’s disease. Many, if not most, FTD suffers are misdiagnosed and are often treated for psychiatric disorders, depression, or Alzheimer’s itself.
What’s wrong with Jacques? Exploring frontotemporal dementia
By: Conor Burke in Clinical Focus, Top Stories October 24, 2019
https://www.agedcareinsite.com.au/2019/10/whats-wrong-with-jacques-exploring-frontotemporal-dementia/
Olivier Piguet knew something was wrong with his cousin Jacques. During a phone conversation, Olivier’s mother mentioned that Jacques, then a successful and well-liked optometrist in his local Swiss town, was acting peculiar. Suddenly rude to customers and falling out with his business partner, he was struggling to keep the business running. Olivier told his mother that this sounded like the early symptoms of frontotemporal dementia (FTD). This would have been classed as an amazing feat of diagnostic assessment were it not for the fact that Olivier had spent his life studying the disease as a research fellow and professor of clinical neuropsychology.
Jacques was lucky that his cousin, thousands of miles away in Australia, was familiar with these symptoms, as FTD can often go misdiagnosed for years, especially in people under 65 – like Jacques, then aged 49. FTD can often be diagnosed as depression or other mental illness as the initial symptoms can be similar.
“When some someone starts developing these symptoms, you don’t think dementia because dementia is something that happens in old age,” Piguet says.
“If you start developing these symptoms in your 30s, 40s, it takes about five years to get to a diagnosis. When you’re in your 70s it takes about half as long to get a diagnosis of dementia. So, 18 months, two years.”
FTD is a form of dementia that is a result of damage to the frontal and/or lobal areas of the brain, the area that is important for controlling voluntary behaviour, emotions and complex thought. Although FTD is a less common disease, approximately 15–20 per cent of all cases of dementia will have some FTD-related syndromes. It most commonly affects people in their 50s or 60s, compared with Alzheimer’s disease which manifests at a later age.
FTD can be divided into three sub-categories (although these sometimes overlap):
There is the behavioural variant and two language presentations –
- semantic dementia and
- progressive non-fluent aphasia. Symptoms of the behavioural variant can be subtle and not always associated with dementia, hence the difficulty in diagnosis.
- Patients will often become selfish, lose empathy or emotional warmth and responses. They can often lose their inhibitions and have difficulties in judgement, organisation or show impulsiveness.
- The language variants present different challenges.
“Progressive non-fluent aphasia is much more obvious because you have this difficulty with expressive language. They understand fine, but they want to produce the output; that’s where there’s a major problem. So you have all this distorted garbled speech.”
People with FTD will often present with one of the three sub-types initially and then develop other symptoms over time.
The ways we treat FTD are different as well. Unlike other types of dementia, it does not respond to medication, says Piguet.
“In terms of disease modifying treatments like drugs, there’s really nothing in the dementia space. The only drugs that are available will essentially boost the efficiency of the nerve cells that are left in the brain. So like drinking 10 cups of coffee essentially – it makes you more buzzy and aware. It helps these nerve cells work as best as they can, but it doesn’t stop the disease process. So progressively these nerve cells will still die, and the medications will stop working,” he says.
“In frontotemporal dementia, we don’t even have that. All the drug trials that have been going on in the past few years have been negative. One reason is because of these multiple pathologies – a drug is likely to target on one of these protein accumulations, not all of them.” However, like Alzheimer’s, there are ways in which people with FTD can train and exercise their brains to allay symptoms and slow decline. Attempting to alleviate behaviours is important for the carers and families of those with FTD.
- - - - - - - - - - - - - - - - - - - - - - - - - - - - - -
FTD refers to a group of rare neurological disorders mostly affecting the frontal and anterior temporal lobes of the brain.
These areas control “executive functions” such as
- reasoning,
- personality,
- social behavior,
- movement,
- speech,
- language,
- and certain aspects of memory.
FTD usually develops between the ages of 35 and 75 affecting men and women about equally. FTD is believed to make-up only 3% of all dementia cases.
Some of the diseases classified as FTD include:
- Pick’s Disease,
- FEDP-17,
- Supranuclear Palsy,
- Primary Progressive Aphasia and
- Corticobasal Degeneration.
The frontal and temporal lobes handle the executive and language functions of the brain. When these brain regions are affected, many of the brain’s communication, sorting and coordination functions are impacted. This means that disability, behaviour disturbance and risk can be more severe in earlier stages of FTD than in other forms of dementia.
People with hyperactive or ritualistic behavioural presentations of FTD are often excluded or rejected from planned activity programs or social gatherings for people with dementia, because of a perceived inability to benefit or because their symptoms disrupt others in the group. It is important to consider FTD as a focal dementia, meaning that more medial and posterior functions of the brain such as memory and visual/spatial skills may be relatively spared for much of the disease course.
Research by neuroscientists at the University of Sydney has shown that people living with frontotemporal dementia – a form of younger-onset dementia – lose the ability to daydream and have no inner monologue. It is believed that those with healthy minds spend at least 50% of their waking lives daydreaming and allowing their mind to wander, and this train of internal thought has many positive benefits for overall mental health.
01 March 2019
Introspective thinking and daydreaming allow a person to analyse their own behavior and the behaviors of others as they reflect on past events and plan for future events in their mind. This type of thinking is one of the main catalysts for emotional and behavioral regulation and it is also associated with problem-solving skills and personal creativity.
Professor Muireann Irish, Associate Professor Brain and Mind Centre and School of Psychology at the University of Sydney noticed many interesting things throughout the study, but even though the study was obviously informative, the findings did reveal an upsetting reality. “Unfortunately, one of the things that we took from the study was just how sad these findings really are. Imagine not having the ability to daydream and escape the present moment. Imagine how limiting that is to a person,” said Professor Irish.
An FTD specific program may place more emphasis on short activities that require less concentration, activities that burn off excess energy and agitation with physical activity and more meaningful activities for a younger cohort. Similarly individualized assessment may find ways to engage individuals with symptoms of apathy.
How is carer burden different in FTD?
Carers of individuals with FTD are an often overlooked group. Importantly, carers are at risk of experiencing physical, emotional, and social difficulties while caring for someone with a condition such as FTD (Schulz and Martire 2004). The caregiver burden in FTD is greater than in other forms of dementia such as AD (Piguet, Hornberger et al. 2011), and is possibly related to the unique behavioural and cognitive symptoms of FTD. People with FTD often lose insight early in their disease process, creating significant barriers to the person accepting they have a problem, their willingness to seek diagnosis and to accept they need help and support. This greatly contributes to tensions in communication and care delivery as the person with FTD may resist support.
People with FTD will often lose decision making capacity and planning skills early and so carers of people with FTD tend to be supporting these functions for longer than in other types of dementia.
The U.S based website: www.ftdsupport.com offers an international chat forum for FTD carers specifically. Alzheimer’s Australia Vic has also evaluated a pilot chat forum for people with younger onset dementia and their carers.
FTD Online Support Forum is at http://www.ftdsupportforum.com/
The purpose of this online forum is to provide a place for safe and secure communications for people who have been diagnosed with FTD and those who care for loved ones with FTD.
Members are dedicated to supporting one another in a sensitive, respectful and sincere manner.
This forum also encourages increased awareness of FTD within the medical community and the general public, with the belief that knowledge will bring improved access to support networks that are emerging on the internet and elsewhere.
http://www.theaftd.org.au/about-afd/
Download toolkits at https://ecdc.org.au/ftd-toolkit
Note: by clicking on each heading, there is a relevant dropdown with a .pdf to download.
https://www.dementia.org.au/information/about-dementia/types-of-dementia/frontotemporal-dementia
The right and left frontal lobes at the front of the brain are involved in
- mood,
- social behaviour,
- attention,
- judgement,
- planning and self-control.
- Damage can lead to reduced intellectual abilities and changes in personality, emotion and behaviour.
The right and left temporal lobes at the two sides of the brain are involved in processing what we hear and understanding what we hear and see. Damage may lead to difficulty recognising objects or understanding or expressing language.
FTD is sometimes called frontotemporal lobar degeneration. It was first described 100 years ago by Arnold Pick and was previously referred to as Pick’s disease.
The symptoms of FTD depend on which areas of the brain are damaged. In contrast to Alzheimer’s disease, memory often remains unaffected in FTD, especially in the early stages. When the frontal lobes are affected first, the main changes are in personality and behaviour, and this is called behavioural-variant FTD. When the temporal lobes are affected first, there is a loss of language skills. There are two types of FTD where language is impaired – progressive nonfluent aphasia and semantic dementia.
Behavioural-variant frontotemporal dementia
In the frontal or behavioural variant of FTD, there are changes in the person’s behaviour, habits, personality and/or emotional responses. Symptoms vary from person to person depending on which areas of the frontal lobes are damaged. Some people with behavioural-variant FTD become very apathetic, while others become disinhibited.
Common symptoms can include:
- Fixed mood and behaviour, appearing selfish and unable to adapt to new situations
- Loss of empathy, emotional warmth and emotional responses
- Apathy or lack of motivation, abandoning hobbies or avoiding social contact
- Loss of normal inhibitions, talking to strangers or exhibiting embarrassing behaviour
- Difficulty in reasoning, judgement, organisation and planning
- Distractibility and impulsiveness
- Changes in eating patterns, craving sweet foods, overeating or unusual food preferences
- A decline in self-care and personal hygiene
Reduced ability to perform daily activities is an early feature. As the disease progresses, the person may become obsessive, or repeat patterns of movement or behaviours.
The Australian Fronto-Temporal Dementia Association (AFTDA)
Overlap between frontotemporal dementia and movement disorders
Most people with FTD do not experience significant movement or motor problems. However, motor symptoms can develop in the later stages of the illness and FTD can co-occur with other conditions characterised by motor symptoms.
These include motor neuron disease, corticobasal syndrome, progressive supranuclear palsy and FTDP-17 (FTD with parkinsonism linked to chromosome 17).
Is there treatment available?
There is not yet a cure for FTD, or any diseasemodifying treatment. However, various therapies can help with some of the symptoms. Secondary symptoms, such as depression, may be helped by medication.
Knowing more about FTD and why the person is behaving as they are can help people to cope with the disease.
Family members and carers can, with support, develop coping strategies to work around problems rather than trying to change the behaviour of the person with FTD.
Click here for information - like how to find them :-) on the following therapies:
Psychological therapies are important to help manage abnormal behaviour.
Speech therapy is of benefit to people with PNFA, particularly in helping to develop alternative communication methods.
The input of an Occupational therapist can help improve everyday functioning at home.
8 November 2019